Acquired pure red cell aplasia in Japan

Abstract

We reviewed the clinical features of 150 patients with acquired pure red cell aplasia (PRCA) in Japan. There were 35 patients with acute type and 115 with chronic type PRCA. Of the acute PRCA patients, 17 had human parvovirus B19 infection. Drug‐induced PRCA was demonstrated in 7 patients. Of the 115 patients with chronic PRCA, 51 patients were classified as primary and 64 cases were associated with miscellaneous diseases such as thymoma, a variety of hematological disorders and collagen diseases. Among the hematological disorders, PRCA was most frequently seen in granular lymphocyte proliferative disorders (GLPD). The erythroid colony growth patterns from bone marrow were variable. The serum erythropoietin level was high in most patients. Various kinds of treatment were tried for the chronic PRCA cases. Cyclosporin A (CyA) was the most effective form of treatment and the response rate was 82% (31/38). Twenty‐three of 37 patients (62%) responded to bolus methylprednisolone therapy. The largest number of patients were treated with oral prednisolone, and the therapy was effective in 27 of the 55 (49%). The response rate to cyclophosphamide was only 29% (5/17), but in combination with prednisolone, half of the patients (7/14) responded to the therapy. CyA is recommended as the first‐line therapy for acquired chronic PRCA.

Long-term responses and outcomes following immunosuppressive therapy in large granular lymphocyte leukemia-associated pure red cell aplasia: a Nationwide Cohort Study in Japan for the PRCA Collaborative Study Group

“…[1][2][3] LGL leukemia is also referred to as granular lymphocyte-proliferative disorders (GLPD) or lymphoproliferative disease of granular lymphocytes. 1,2,[4][5][6] LGL leukemia is a heterogeneous disorder characterized by a persistent increase in the number of peripheral blood LGLs, and the majority of patients have a clonal rearrangement of T-cell receptors. 4,6,7 Clonal disorders of LGLs may arise from natural killer cells, and may be indolent or behave as an aggressive disease.…”

Pure red-cell aplasia as the presenting feature of the carcionoid tumor of the thymus: case report

“…Although surgical resection of the tumor is recommended it does not usually lead to a remission in the anemia, even in patients cured of their tumors [3,9,10]. The response rates to thymectomy are reported to be 12%-30% [4,10] suggesting that the thymic tumor may not be directly involved in the pathogenesis of the PRCA; rather both these conditions may arise as a consequence of another, currently unknown, pathology.…”

“…The response rates to thymectomy are reported to be 12%-30% [4,10] suggesting that the thymic tumor may not be directly involved in the pathogenesis of the PRCA; rather both these conditions may arise as a consequence of another, currently unknown, pathology. Various immunosuppressive treatment modalities have been attempted with variable efficacies (these include corticosteroids, cyclosporine A, cytotoxic agents, antithymocyte globulin, danazol, rituximab, and splenectomy) [5,7,9,10].…”

Thymoma with Concomitant Pure Red Cell Aplasia, Good’s Syndrome and Myasthenia Gravis Responding to Rituximab

“…Thymectomy also represents the first-line of therapy for myasthenia gravis-associated with thymomas before pharmacologic therapies are instituted [11]. Thymectomy combined with immunoglobulin replacement represents the mainstay in the treatment of concomitant Good's syndrome and thymomas [12]. In our particular case, patient's age and associated comorbidities as well as the critical anatomic location of his tumor precluded the option of surgery.…”